ESPE Abstracts

Background: GH treatment is effective in improving adult height (AH) in short children born Small for Gestational Age. If SGA children are short at the start of puberty, they could benefit from combined GH/GnRH Analogue (GnRHa) treatment.Objective and hypotheses: To determine the timing and progression of pubertal development in short SGA children, comparing GH treatment with combined GH/GnRHa treatment.Method: For the present stud...

Background: Bilateral adrenal haemorrhage is rare in the neonates and more rarely does it manifest itself as acute adrenal insufficiency (AI). Cytomegalovirus (CMV)-associated AI is a well-known in adults with acquired immunodeficiency syndrome. AI is not a common finding in children with congenital CMV infection. We describe herein the case of a newborn infant presenting with adrenal hematomas (AH), AI and congenital CMV infection.Case report: A 20 day-...

Background: In a previous study, we reported that adult height (AH) outcomes in growth hormone treated males with Silver-Russell syndrome (SRS) were negatively correlated with estradiol concentrations before start of puberty and in early puberty. Whether elevated estradiol concentrations originated from adrenal or testicular androgens is unclear. We aimed to describe androgen secretion patterns and investigate correlations between androgen concentrations and A...

Background: Pubertal children born small for gestational age (SGA) with a poor adult height (AH) expectation can benefit from treatment with GH 1 mg/m2 per day (~0.033 mg/kg per day) in combination with 2 years of GnRH analogue (2 years GnRHa) and even more so with 2 mg/m2 per day (~0.067 mg/kg per day). Concerns haven been raised about the effects of GH and GnRHa on insulin sensitivity on the long-term.Objective and hypotheses: To ...

Background: Due to fewer girls than boys being GH-treated, after 50yrs the knowledge about sex-differences in both GH-response and GH-responsiveness before and during puberty remains limited.Aim: To describe factors explaining growth response to GH-treatment, separately in girls and boys from GHstart to adult height (AH).Methods: Children diagnosed with IGHD/ISS/SGA from GH-SAFETY-...

Background: severe primary insulin-like growth factor-1 deficiency (SPIGFD) is a rare growth disorder. Recombinant human insulin-like growth factor (IGF-1) (rhIGF-1; Increlex® [mecasermin]) replacement therapy is EU and US-approved for treating growth failure due to SPIGFD. The long-term therapeutic objective of rhIGF-1 treatment in SPIGFD is to improve adult height (AH). Objective: to describe the characteristics, safety and effectiveness data ...

Context: In children with Prader-Willi syndrome (PWS), the benefits of growth hormone (GH) treatment are well established. Currently, when young adults with PWS have attained adult height (AH), they have to stop GH treatment. Several one year studies have shown that GH treatment is also beneficial for adults with PWS, improving body composition. However, little is known about the longer-term effects.Objective: To investi...

Introduction: The prevalence of obesity constantly increases worldwide and definitely increases the risk of premature death in early adulthood. Metabolic dysfunction-associated fatty liver disease (MAFLD) – a new term for fatty liver disease accompanied by other components of metabolic syndrome (MetS) connected with increase cardiovascular risk either in adult or children. Whereas there is no yet treatment with proven efficacy for the metabolic clamp suc...

Background: Being born small for gestational age (SGA) has a negative effect on health-related quality of life (HRQoL) and self-perception. This might be more negatively influenced by postponement of puberty using additional gonadotropin-releasing hormone analogue (GnRHa) treatment.Methods: 154 adolescents born SGA participating in a large Dutch growth hormone (GH) trial (75 with 2 years of GnRHa-treatment) completed the TNO-AZL Adults Quality of Life qu...

Background: In children born SGA with persistent short stature, growth hormone (GH) treatment is an approved therapy for increasing adult height (AH). Some SGA children have an advanced bone age (BA) during GH. Heterozygous mutations in the ACAN-gene have been described in children with idiopathic short stature and advanced BA.Objective and hypotheses: To assess growth during GH treatment with additional GnRHa treatment, in children with ACAN-gene mutati...